Pathological Findings of IgG4-Related Lung Disease

  • Shoko Matsui
  • Kenji Notohara
  • Yuko Waseda


IgG4-related disease (IgG4-RD) causes temporally and spatially diverse lesions in systemic organs, with respiratory organ involvement now known to include the bronchi, bronchioles, alveolar wall, pleura, pulmonary vessels, and mediastinal lymph nodes. The histopathological features of the lesion site are a marked lymphoplasmacytic infiltrate, diffusely distributed IgG4-positive plasma cells, and obliterative inflammation of arteries and/or veins. Some non-neoplastic lung diseases, whose cause was hitherto unknown, may be encompassed in this disease. Many such disorders must now be reconsidered in light of information emerging about IgG4-RD.


Anaplastic Lymphoma Kinase Interstitial Pneumonia Anaplastic Large Cell Lymphoma Inflammatory Myofibroblastic Tumor Nonspecific Interstitial Pneumonia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



The authors thank Dr. Joanne E. Yi, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA, and Dr. Yasumasa Monobe, Department of Pathology, Kawasaki Hospital, Okayama, Japan, for their kind contribution of some figures for this chapter.


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Copyright information

© Springer Japan 2014

Authors and Affiliations

  1. 1.Health Administration CenterUniversity of ToyamaToyama cityJapan
  2. 2.Department of Anatomic PathologyKurashiki Central HospitalKurashikiJapan
  3. 3.Department of Respiratory MedicineKanazawa University School of MedicineKanazawaJapan

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